Adult-Onset Still’s Disease Presenting as Macrophage-Activation Syndrome With Critical Illness in the Third Trimester of Pregnancy: A Case Report

Peters, Andrew T; Prickett, Michelle

doi:10.1097/cce.0000000000000440

Cited by 4 publications

(3 citation statements)

References 13 publications

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“…The response of the patient to immunosuppressive therapy highlights the significance of considering AOSD in the differential diagnosis of pregnant patients with unexplained fever and multiorgan dysfunction, thereby contributing valuable insights into the potential severe manifestations of AOSD, including pulmonary involvement and systemic inflammation, during pregnancy. 12 Yip et al's study highlights the diagnostic challenges of HLH during pregnancy, emphasizing its symptomatic overlap with common conditions in pregnancy such as fever and liver dysfunction and the importance of considering HLH in the differential diagnoses. In their study, they described a case of a 23-year-old pregnant woman diagnosed with HLH at 22 weeks of gestation.…”

Section: Association With Hemophagocytic Lymphohistiocytosis (Hlh)mentioning

confidence: 99%

Section: Association With Hemophagocytic Lymphohistiocytosismentioning

confidence: 99%

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Pregnancy-Associated Onset of Adult-Onset Still's Disease

Zayat,

Arora,

O'Brien

et al. 2024

AJP Rep

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Objective: This study aims to elucidate the clinical manifestations, diagnostic challenges, and management strategies of Adult-Onset Still’s Disease (AOSD) during pregnancy, leveraging a case series overview and a detailed case report from our center. Study Design: A comprehensive review of 21 published case reports on AOSD diagnosed during pregnancy was conducted, alongside a detailed case report of a patient diagnosed and managed at our center. This study emphasizes the importance of recognizing AOSD in pregnant patients, outlines the therapeutic challenges encountered, and discusses the potential complications arising from the disease and its treatment. Results: The onset of AOSD during pregnancy predominantly occurs in the first or second trimester, with a polycyclic disease course observed in the majority of cases. Management primarily involves corticosteroids and immunosuppressive medications. The case report highlights the complex interplay between AOSD, Hemophagocytic Lymphohistiocytosis (HLH), and pregnancy, illustrating a multidisciplinary approach to management that ensured favorable maternal and fetal outcomes despite the significant challenges. Conclusion: AOSD presents unique diagnostic and therapeutic challenges during pregnancy, requiring careful consideration of maternal and fetal health. Early diagnosis, a multidisciplinary approach to care, and judicious use of immunosuppressive therapy are critical for managing AOSD flares and associate

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Section: Association With Hemophagocytic Lymphohistiocytosis (Hlh)mentioning

confidence: 99%

Section: Association With Hemophagocytic Lymphohistiocytosismentioning

confidence: 99%

Pregnancy-Associated Onset of Adult-Onset Still's Disease

Zayat,

Arora,

O'Brien

et al. 2024

AJP Rep

View full text Add to dashboard Cite

show abstract

“…Macrophage activation syndrome is a type of HLH that most often coexists with rheumatic diseases, such as Still's disease, systemic lupus erythematosus, dermatomyositis, juvenile idiopathic arthritis (JIA) or systemic sclerosis. Despite the fact that the complete pathways are unknown, decreased natural killer (NK) cell activity, elevated soluble interleukin-2 receptor (sIL-2R), and broadly excessive cytokine production appear to be elements of MAS pathogenesis [51]. Rheumatic diseases can have different courses during pregnancy.…”

Section: The Course Of Hlh In Pregnant and Postpartum Womenmentioning

confidence: 99%

Cytokine Storms in the Course of COVID-19 and Haemophagocytic Lymphohistiocytosis in Pregnant and Postpartum Women

et al. 2021

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The term ‘cytokine storm’ (CS) applies to a pathological autoimmune reaction when the interactions that lead to cytokine production are destabilised and may even lead to death. CS may be induced by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. In this study, we present our analysis of certain pathological processes that induce a CS in pregnant and postpartum women. We draw our attention to the similarities between the severe course of Coronavirus Disease 2019 (COVID-19) and haemophagocytic lymphohistiocytosis (HLH). It is noteworthy that many of the criteria used to diagnose HLH are described as COVID-19 mortality predictors. Cytokine storms are considered to be an important cause of death in patients with the severe course of SARS-CoV-2 infection. Due to the fact that pregnant women are in an immunosuppressive state, viral pulmonary infections are more perilous for them—possible risks include miscarriage, intrauterine growth restriction or birth before the term; sometimes ventilation support is needed. HLH should be considered in pregnant and puerperal women suffering from moderately severe to severe COVID-19 and presenting with: fever unresponsive to antibiotic therapy, cytopenia, hepatitis and hyperferritinaemia. The HLH disorder is rare and difficult to diagnose; however, its early detection could reduce patient mortality.

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Adult-onset Still’s disease complicated by macrophage activation syndrome during pregnancy: a case-based review

Wise

Zell

2023

Clin Rheumatol

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Adult-onset Still’s disease is a rare, systemic inflammatory rheumatic disease characterized by recurrent fevers, arthritis, and an evanescent rash. One of the most serious hematologic derangements that can be seen with adult-onset Still’s disease is macrophage activation syndrome. Macrophage activation syndrome is characterized by activation of lymphocytes, resulting in a cytokine storm and hemophagocytosis in the bone marrow, along with multi-organ failure. Adult-onset Still’s disease with macrophage activation syndrome first presenting during pregnancy is exceptionally rare; here, we report two unique cases of such a presentation and review the pertinent literature. Both of our cases presented critically ill with end-organ failure, and responded to immunosuppression; fetal demise was present in one and an emergency caesarean section with a viable fetus was performed in the other patient. Maternal outcomes were favorable in both cases and both patients did well long-term with systemic therapy. Systemic immunosuppression, particularly anti-IL1 therapy, may be considered as treatment for this rare and life-threatening condition when presenting during pregnancy.

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Adult-Onset Still’s Disease Presenting as Macrophage-Activation Syndrome With Critical Illness in the Third Trimester of Pregnancy: A Case Report

Abstract: Supplemental Digital Content is available in the text.

Cited by 4 publications

References 13 publications

Pregnancy-Associated Onset of Adult-Onset Still's Disease

Pregnancy-Associated Onset of Adult-Onset Still's Disease

Cytokine Storms in the Course of COVID-19 and Haemophagocytic Lymphohistiocytosis in Pregnant and Postpartum Women

Adult-onset Still’s disease complicated by macrophage activation syndrome during pregnancy: a case-based review

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